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FRCPath Haem Part 1 MCQ-Oncology 384

A 28yr-old pt present with B/L cervical LNs and weight loss. Biopsy-dilated sinuses filled with large lymphocyte aggregate. S100+CD68+CD1a-. Diagnosis?

  • Langerhans cell histiocytosis

  • Hodgkin lymphoma

  • Mastocytosis

  • Erdheim Chester Disease


 
 
 

1 Comment

adwaan567
3 hours ago

Correct Answer: Rosai–Dorfman disease 


Key Features Supporting This Diagnosis:

  • Bilateral cervical lymphadenopathy ✔️

  • Weight loss ✔️ (systemic symptoms can occur)

  • Dilated sinuses filled with large cells/aggregates ✔️

  • Immunohistochemistry:

    • S100 positive ✔️

    • CD68 positive ✔️ (histiocyte marker)

    • CD1a negative ✔️ (rules out Langerhans cell histiocytosis)

Typical Pathology in Rosai-Dorfman Disease:

  • Proliferation of histiocytes in lymph node sinuses

  • Emperipolesis (lymphocytes inside histiocytes) – classic feature

  • Histiocytes are:

    • S100+

    • CD68+

    • CD1a−

Differential (Why Not Others?):

  • Langerhans cell histiocytosis → CD1a+ (not here)

  • Lymphoma → different immunophenotype

  • Tuberculosis → granulomas, not this profile

✅ Final Diagnosis:

Rosai–Dorfman disease (Sinus histiocytosis with massive lymphadenopathy)

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